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沃尔夫管异常的磁共振成像评估——OHVIRA和OSVIRA。

Magnetic Resonance Imaging Evaluation of Wolffian Duct Anomalies - OHVIRA and OSVIRA.

作者信息

Bansal Lukshay, Arora Suryansh, Prasad Akhila

机构信息

Department of Radiodiagnosis, Atal Bihari Vajpayee Institute of Medical Sciences and Dr. Ram Manohar Lohia Hospital, New Delhi, India.

出版信息

J Hum Reprod Sci. 2024 Apr-Jun;17(2):128-132. doi: 10.4103/jhrs.jhrs_19_24. Epub 2024 May 28.

Abstract

Herlyn-Werner-Wunderlich syndrome in females, also known as obstructed haemivagina and ipsilateral renal agenesis (OHVIRA), and Zinner's syndrome, also known as OSVIRA in males, are two rare congenital syndromes affecting the urogenital tract. OHVIRA is obstructed haemivagina and ipsilateral renal agenesis and OSVIRA abbreviation stands for obstructed seminal vesicle and ipsilateral renal agenesis. For a long, these syndromes were considered different entities, owing to our poor understanding of underlying embryopathogenesis; however, in light of the recent acceptance of the Wolffian origin of the vagina in its entirety, these two syndromes are considered to be cut from the same cloth. In this case series, we present magnetic resonance imaging features of two cases each of OHVIRA and OSVIRA and discuss the similarities in embryopathogenesis behind them while debunking the old concepts. A deep understanding of embryopathogenesis allows a clinician and radiologist to predict the disease manifestation, and its outcome and also acts as a guide to screen for potential subtle defects, which would otherwise have been missed.

摘要

女性的赫林-韦纳-温德利希综合征,也称为梗阻性半阴道并同侧肾缺如(OHVIRA),以及男性的津纳综合征,也称为梗阻性精囊并同侧肾缺如(OSVIRA),是两种影响泌尿生殖道的罕见先天性综合征。OHVIRA指梗阻性半阴道并同侧肾缺如,OSVIRA的缩写代表梗阻性精囊并同侧肾缺如。长期以来,由于我们对潜在胚胎发病机制的了解不足,这些综合征被认为是不同的实体;然而,鉴于最近完全接受阴道起源于中肾管这一观点,这两种综合征被认为是同一类情况。在本病例系列中,我们展示了OHVIRA和OSVIRA各两例的磁共振成像特征,并讨论其背后胚胎发病机制的相似性,同时破除旧观念。对胚胎发病机制的深入理解使临床医生和放射科医生能够预测疾病表现及其结果,并且还可作为筛查潜在细微缺陷的指南,否则这些缺陷可能会被漏诊。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9259/11290712/a3df3a0c1f57/JHRS-17-128-g001.jpg

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